Lingual Muscle Training in Late-Onset Pompe Disease (LOPD)

Inclusion criteria:

- Age ≥ 18 years

- Lingual weakness as measured by quantitative muscle testing (≤ 5% lower limit of
normal for stratified age)

- Confirmed diagnosis of LOPD and followed at Duke for this condition

- On enzyme replacement therapy for ≥ 26 weeks at pretest

- Able to follow directions for study participation

- Able to complete a home-based LMT regimen

- Access to reliable internet connection

- Access to use of electronic device that allows for FaceTime, Jabber, or WebEx use

Exclusion Criteria:

- Neurodegenerative conditions (e.g. stroke, dementia) or other serious neurologic
condition that would prevent meaningful study participation as determined at the
discretion of the principle investigator

- Any current or past history of seizures.

- Infantile onset Pompe disease

- Head and neck cancer or radiation treatment to head/neck

- Inability to tolerate IOPI device tongue bulb

- Inability to give legally effective consent

- Inability to read and understand English